Scleroderma

Scleroderma (systemic sclerosis) is an autoimmune condition characterized by diffuse collagen deposition and fibrosis. The clinical presentation varies from limited skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin involvement to diffuse involvement of internal organs. Diagnosis is established by a combination of physical findings and serology. There is no curative treatment. Management options are limited and include immunosuppressive medications as well as specific organ- or symptom-directed drugs. The overall 5-year survival of patients with scleroderma is about 80%.

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Overview

Definition

Scleroderma, also known as systemic sclerosis (SS), is an autoimmune disorder in which there is progressive deposition of collagen in the skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin and internal organs causing tightening and fibrosis.

Epidemiology

  • Women are 5 times more likely to be affected than men.
  • Women more likely to present:
    • Younger
    • With limited disease
    • With pulmonary hypertension Pulmonary Hypertension Pulmonary hypertension (PH) or pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary arterial pressure, which can lead to chronic progressive right heart failure. Pulmonary hypertension is grouped into 5 categories based on etiology, which include primary PAH, and PH due to cardiac disease, lung or hypoxic disease, chronic thromboembolic disease, and multifactorial or unclear etiologies. Pulmonary Hypertension
  • Peak age at presentation: 35–50 years
  • More severe in African American patients

Etiology

  • Etiology is unknown.
  • Genetics Genetics Genetics is the study of genes and their functions and behaviors. Basic Terms of Genetics likely play a role.
  • Suspected environmental triggers include:
    • Infectious ( cytomegalovirus Cytomegalovirus CMV is a ubiquitous double-stranded DNA virus belonging to the Herpesviridae family. CMV infections can be transmitted in bodily fluids, such as blood, saliva, urine, semen, and breast milk. The initial infection is usually asymptomatic in the immunocompetent host, or it can present with symptoms of mononucleosis. Cytomegalovirus (CMV), herpesvirus, parvovirus)
    • Environmental:
      • Silica exposure
      • Solvents (vinyl chloride, benzene)
    • Drugs (bleomycin)
    • Radiation exposure

Pathophysiology and Clinical Presentation

Pathophysiology

  • Not completely understood
  • 3 interlinked complex processes are the basis for pathogenesis:
    • Abnormal activation of both humoral and cell immunity → autoimmune disorder
      • Microvascular endothelial injury likely caused by anti-endothelial antibodies Antibodies Immunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution. Immunoglobulins
      • Perivascular accumulation of T and B lymphocytes B lymphocytes B lymphocytes, also known as B cells, are important components of the adaptive immune system. In the bone marrow, the hematopoietic stem cells go through a series of steps to become mature naive B cells. The cells migrate to secondary lymphoid organs for activation and further maturation. B Cells, macrophages, and fibroblast precursors
      • Secretion of cytokines and growth factors by inflammatory cells
    • Abnormal stimulation of fibroblast activation and proliferation within small arteries Arteries Arteries are tubular collections of cells that transport oxygenated blood and nutrients from the heart to the tissues of the body. The blood passes through the arteries in order of decreasing luminal diameter, starting in the largest artery (the aorta) and ending in the small arterioles. Arteries are classified into 3 types: large elastic arteries, medium muscular arteries, and small arteries and arterioles. Arteries and arterioles 
    • Progressive deposition of collagen and other extracellular matrix molecules within the skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin and internal organs

Clinical variants

  • Diffuse cutaneous SS: 
    • Puffy hands
    • Involves skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin proximal to the knees and elbows (trunk, neck, and face)
    • Early and extensive involvement of internal organs in the course of the disease
    • Rapid progression with diffuse skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin thickening and lung, renal, and cardiac involvement
  • Limited cutaneous SS:
    • Puffy fingers distal to metacarpophalangeal joints
    • Limited to skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin distal to elbows and knees
    • Prominent vascular symptoms:
      • Raynaud’s phenomenon
      • Telangiectasias
      • Pulmonary arterial hypertension (PAH; late symptom)
    • Also referred to as CREST syndrome:
      • Calcinosis
      • Raynaud’s phenomenon
      • Esophageal dysmotility 
      • Sclerodactyly (tight skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin over digits)
      • Telangiectasias 
  • SS sine scleroderma (rare):
    • No detectable skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin involvement
    • Raynaud’s phenomenon, digital ulcers, and PAH
  • SS with overlap syndrome:
    • Symptoms of any of the other subsets
    • Overlap with other rheumatologic diseases:
      • Lupus
      • Rheumatoid arthritis Rheumatoid arthritis Rheumatoid arthritis (RA) is a symmetric, inflammatory polyarthritis and chronic, progressive, autoimmune disorder. Presentation occurs most commonly in middle-aged women with joint swelling, pain, and morning stiffness (often in the hands). Rheumatoid Arthritis
      • Polymyositis Polymyositis Polymyositis (PM) is an autoimmune inflammatory myopathy caused by T cell-mediated muscle injury. The etiology of PM is unclear, but there are several genetic and environmental associations. Polymyositis is most common in middle-aged women and rarely affects children. Polymyositis
      • Sjögren’s syndrome

Clinical symptoms

Diffuse and limited cutaneous SS:

  • Constitutional symptoms:
    • Fever Fever Fever is defined as a measured body temperature of at least 38°C (100.4°F). Fever is caused by circulating endogenous and/or exogenous pyrogens that increase levels of prostaglandin E2 in the hypothalamus. Fever is commonly associated with chills, rigors, sweating, and flushing of the skin. Fever
    • Fatigue
    • Weight loss 
  • Skin:
    • Swelling with non-pitting edema Edema Edema is a condition in which excess serous fluid accumulates in the body cavity or interstitial space of connective tissues. Edema is a symptom observed in several medical conditions. It can be categorized into 2 types, namely, peripheral (in the extremities) and internal (in an organ or body cavity). Edema
    • Skin thickening/tightening:
      • Sclerodactyly 
      • Perioral skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin → reduced mouth opening
      • Around small joints → contractures
    • Hyper-/hypopigmentation
    • Pruritus
  • Hands:
    • Prolonged Raynaud’s phenomenon
    • Calcinosis cutis (calcium deposition under the skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin → hard bumps)
    • Digital ulceration
    • Gangrene
    • Nail bed telangiectasia

Diffuse forms with or without cutaneous involvement:

  • Gastrointestinal (90%):
    • Dysphagia Dysphagia Dysphagia is the subjective sensation of difficulty swallowing. Symptoms can range from a complete inability to swallow, to the sensation of solids or liquids becoming "stuck." Dysphagia is classified as either oropharyngeal or esophageal, with esophageal dysphagia having 2 sub-types: functional and mechanical. Dysphagia
    • Gastroesophageal reflux disease Gastroesophageal Reflux Disease Gastroesophageal reflux disease (GERD) occurs when the stomach acid frequently flows back into the esophagus. This backwash (acid reflux) can irritate the lining of the esophagus, causing symptoms such as retrosternal burning pain (heartburn). Gastroesophageal Reflux Disease
    • Hoarseness
    • Malabsorption Malabsorption Malabsorption involves many disorders in which there is an inability of the gut to absorb nutrients from dietary intake, potentially including water and/or electrolytes. A closely related term, maldigestion is the inability to break down large molecules of food into their smaller constituents. Malabsorption and maldigestion can affect macronutrients (fats, proteins, and carbohydrates), micronutrients (vitamins and minerals), or both. Malabsorption and Maldigestion
    • Vascular ectasia → watermelon stomach Stomach The stomach is a muscular sac in the upper left portion of the abdomen that plays a critical role in digestion. The stomach develops from the foregut and connects the esophagus with the duodenum. Structurally, the stomach is C-shaped and forms a greater and lesser curvature and is divided grossly into regions: the cardia, fundus, body, and pylorus. Stomach
  • Pulmonary (80%):
    • Interstitial lung disease ( ILD ILD Interstitial lung diseases are a heterogeneous group of disorders characterized by the inflammation and fibrosis of lung parenchyma, especially the pulmonary connective tissue in the alveolar walls. It may be idiopathic (e.g., idiopathic pulmonary fibrosis) or secondary to connective tissue diseases, medications, malignancies, occupational exposure, or allergens. Interstitial Lung Diseases) → pulmonary fibrosis Pulmonary Fibrosis Idiopathic pulmonary fibrosis is a specific entity of the major idiopathic interstitial pneumonia classification of interstitial lung diseases. As implied by the name, the exact causes are poorly understood. Patients often present in the moderate to advanced stage with progressive dyspnea and nonproductive cough. Pulmonary Fibrosis
    • Pulmonary arterial hypertension 
    • Pulmonary embolism Pulmonary Embolism Pulmonary embolism (PE) is a potentially fatal condition that occurs as a result of intraluminal obstruction of the main pulmonary artery or its branches. The causative factors include thrombi, air, amniotic fluid, and fat. In PE, gas exchange is impaired due to the decreased return of deoxygenated blood to the lungs. Pulmonary Embolism
  • Cardiac:
    • Restrictive cardiomyopathy Restrictive Cardiomyopathy Restrictive cardiomyopathy (RCM) is a fairly uncommon condition characterized by progressive stiffening of the cardiac muscle, which causes impaired relaxation and refilling of the heart during diastole, resulting in diastolic dysfunction and eventual heart failure. Restrictive Cardiomyopathy
    • Constrictive pericarditis Pericarditis Pericarditis is an inflammation of the pericardium, often with fluid accumulation. It can be caused by infection (often viral), myocardial infarction, drugs, malignancies, metabolic disorders, autoimmune disorders, or trauma. Acute, subacute, and chronic forms exist. Pericarditis
    • Arrhythmias 
  • Genitourinary:
    • Dyspareunia
    • Erectile dysfunction Erectile Dysfunction Erectile dysfunction (ED) is defined as the inability to achieve or maintain a penile erection, resulting in difficulty to perform penetrative sexual intercourse. Local penile factors and systemic diseases, including diabetes, cardiac disease, and neurological disorders, can cause ED. Erectile Dysfunction
  • Renal:
    • Hypertension Hypertension Hypertension, or high blood pressure, is a common disease that manifests as elevated systemic arterial pressures. Hypertension is most often asymptomatic and is found incidentally as part of a routine physical examination or during triage for an unrelated medical encounter. Hypertension
    • Chronic kidney disease Chronic Kidney Disease Chronic kidney disease (CKD) is kidney impairment that lasts for ≥ 3 months, implying that it is irreversible. Hypertension and diabetes are the most common causes; however, there are a multitude of other etiologies. In the early to moderate stages, CKD is usually asymptomatic and is primarily diagnosed by laboratory abnormalities. Chronic Kidney Disease
    • Scleroderma renal crisis:
      • Life-threatening acute renal failure
      • Malignant hypertension
  • Musculoskeletal:
    • Arthralgia/arthritis
    • Joint contractures
    • Tendon friction rubs
    • Tendinitis
  • Neuromuscular:
    • Muscle atrophy
    • Weakness
    • Myopathy
    • Neuropathies

Related videos

Diagnosis

Physical exam

  • 1st step in evaluation
  • Look for characteristic skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin and hand changes.

Laboratory studies

  • General:
    • Complete blood count ( anemia Anemia Anemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology. Anemia: Overview):
      • Malabsorption Malabsorption Malabsorption involves many disorders in which there is an inability of the gut to absorb nutrients from dietary intake, potentially including water and/or electrolytes. A closely related term, maldigestion is the inability to break down large molecules of food into their smaller constituents. Malabsorption and maldigestion can affect macronutrients (fats, proteins, and carbohydrates), micronutrients (vitamins and minerals), or both. Malabsorption and Maldigestion
      • Iron deficiency
      • GI blood loss
    • Creatine kinase: myopathy/myositis
    • Urinalysis to assess kidney function:
      • Proteinuria
      • Cellular casts
    • Serum creatinine
  • Serology:
    • Antinuclear antibody (ANA): present in 95% of patients
    • Anti-centromere antibody: specific for limited SS
    • Anti-topoisomerase (anti-Scl 70): 
      • Specific for diffuse SS
      • Associated with interstitial lung disease
    • Anti- RNA RNA Ribonucleic acid (RNA), like deoxyribonucleic acid (DNA), is a polymer of nucleotides that is essential to cellular protein synthesis. Unlike DNA, RNA is a single-stranded structure containing the sugar moiety ribose (instead of deoxyribose) and the base uracil (instead of thymine). RNA generally carries out the instructions encoded in the DNA but also executes diverse non-coding functions. RNA Types and Structure polymerase III:
      • Specific for diffuse SS
      • Associated with rapidly progressive skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin involvement and scleroderma renal crisis

Additional tests

  • Pulmonary function test (PFT):
    • Restrictive ventilatory defect
    • Decrease in single-breath diffusion capacity for carbon monoxide
  • High-resolution chest computed tomography (CT) scan: interstitial lung abnormalities
  • Echocardiogram: screening for PAH
  • Other studies depending on a particular organ involvement
Scleroderma ct

Chest CT scan showing lung fibrosis at the time of diagnosis of progressive systemic sclerosis

Image: “Chest computed tomography scan” by Division of Respiratory Medicine, Mito Medical Center, University of Tsukuba, Mito, Ibaraki 310-0015, Japan. License: CC BY 3.0

Skin biopsy

  • Rarely indicated
  • Can be performed to differentiate from other diseases (e.g., eosinophilic fasciitis, scleromyxedema, amyloidosis Amyloidosis Amyloidosis is a disease caused by abnormal extracellular tissue deposition of fibrils composed of various misfolded low-molecular-weight protein subunits. These proteins are frequently byproducts of other pathological processes (e.g., multiple myeloma). Amyloidosis)

Management

There is no curative treatment for SS.

Management goals

  • Minimize symptoms.
  • Delay the progression of organ-specific complications.

Skin

  • Pruritus:
    • Prevent dryness (lubricating lotions; avoid drying soaps, heat).
    • Antihistamines Antihistamines Antihistamines are drugs that target histamine receptors, particularly H1 and H2 receptors. H1 antagonists are competitive and reversible inhibitors of H1 receptors. First-generation antihistamines cross the blood-brain barrier and can cause sedation. Antihistamines
    • Low-dose prednisone
  • Skin sclerosis:
    • 1st line: methotrexate or mycophenolate mofetil
    • 2nd line: cyclophosphamide
    • For refractory cases: immune globulin or rituximab
    • Ultraviolet A light therapy also beneficial
  • Cutaneous calcinosis:
    • Oral minocycline
    • Surgical removal of lesions in some cases
  • Telangiectasia: laser therapy

Other organs

  • Kidneys Kidneys The kidneys are a pair of bean-shaped organs located retroperitoneally against the posterior wall of the abdomen on either side of the spine. As part of the urinary tract, the kidneys are responsible for blood filtration and excretion of water-soluble waste in the urine. Kidneys:
    • Angiotensin-converting inhibitors
    • Avoid glucocorticoids Glucocorticoids Glucocorticoids are a class within the corticosteroid family. Glucocorticoids are chemically and functionally similar to endogenous cortisol. There are a wide array of indications, which primarily benefit from the antiinflammatory and immunosuppressive effects of this class of drugs. Glucocorticoids.
  • Esophageal reflux: H2 blockers or proton-pump inhibitors
  • Raynaud’s phenomenon: 
    • Avoid cold.
    • Calcium-channel blockers
    • Prostacyclin analogs
  • Pulmonary hypertension: 
    • Endothelin receptor antagonist (bosentan)
    • Phosphodiesterase inhibitors Phosphodiesterase inhibitors Phosphodiesterase (PDE) inhibitors are a group of drugs that act by inhibiting PDE enzymes. Phosphodiesterase inhibitors have various mechanisms of action depending on the subtype of PDE targeted, but their main action is increasing the amount of intracellular cAMP or cGMP, which in turn results in physiologic effects such as reducing inflammation, promoting smooth muscle relaxation, and vasodilation. Phosphodiesterase Inhibitors (tadalafil)
    • Prostacyclin pathway agonists (epoprostenol)
  • Pulmonary fibrosis: 
    • Mycophenolate mofetil
    • Cyclophosphamide

Prognosis

  • Overall 5-year survival is 80%.
  • Mortality in patients with scleroderma is 4 times higher than in sex- and age-matched controls.
  • Major predictors of mortality:
    • Extensive skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin involvement
    • Lung or cardiac disease
    • Renal disease
    • Younger age of onset
    • African descent

Differential Diagnosis

  • Hypothyroidism Hypothyroidism Hypothyroidism is a condition characterized by a deficiency of thyroid hormones. Iodine deficiency is the most common cause worldwide, but Hashimoto's disease (autoimmune thyroiditis) is the leading cause in non-iodine-deficient regions. Hypothyroidism: a disorder associated with decreased production of thyroid hormones Thyroid hormones The 2 primary thyroid hormones are triiodothyronine (T3) and thyroxine (T4). These hormones are synthesized and secreted by the thyroid, and they are responsible for stimulating metabolism in most cells of the body. Their secretion is regulated primarily by thyroid-stimulating hormone (TSH), which is produced by the pituitary gland. Thyroid Hormones. One manifestation is myxedema, which produces skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin changes similar to SS with coarseness and thickening. Patients also complain of fatigue. Diagnosed by measuring thyroid hormone and thyroid-stimulating hormone levels. The treatment is based on thyroid hormone replacement.
  • Diabetes: an endocrine disorder caused by insulin Insulin Insulin is a peptide hormone that is produced by the beta cells of the pancreas. Insulin plays a role in metabolic functions such as glucose uptake, glycolysis, glycogenesis, lipogenesis, and protein synthesis. Exogenous insulin may be needed for individuals with diabetes mellitus, in whom there is a deficiency in endogenous insulin or increased insulin resistance. Insulin deficiency or insulin Insulin Insulin is a peptide hormone that is produced by the beta cells of the pancreas. Insulin plays a role in metabolic functions such as glucose uptake, glycolysis, glycogenesis, lipogenesis, and protein synthesis. Exogenous insulin may be needed for individuals with diabetes mellitus, in whom there is a deficiency in endogenous insulin or increased insulin resistance. Insulin resistance. Long-standing diabetes mellitus Diabetes mellitus Diabetes mellitus (DM) is a metabolic disease characterized by hyperglycemia and dysfunction of the regulation of glucose metabolism by insulin. Type 1 DM is diagnosed mostly in children and young adults as the result of autoimmune destruction of β cells in the pancreas and the resulting lack of insulin. Type 2 DM has a significant association with obesity and is characterized by insulin resistance. Diabetes Mellitus type 1 may be associated with skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin changes and sclerodactyly. Diagnosis is established by measurement of blood glucose levels. Treatment is focused on insulin Insulin Insulin is a peptide hormone that is produced by the beta cells of the pancreas. Insulin plays a role in metabolic functions such as glucose uptake, glycolysis, glycogenesis, lipogenesis, and protein synthesis. Exogenous insulin may be needed for individuals with diabetes mellitus, in whom there is a deficiency in endogenous insulin or increased insulin resistance. Insulin replacement and blood glucose control.
  • Amyloidosis: a disorder of extracellular tissue deposition of fibrils. Infiltration of the skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin can cause thickness and systemic deposition can result in symptoms very similar to those of diffuse scleroderma. Diagnosis is established by skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin biopsy. Treatment modalities depend on the type of amyloidosis Amyloidosis Amyloidosis is a disease caused by abnormal extracellular tissue deposition of fibrils composed of various misfolded low-molecular-weight protein subunits. These proteins are frequently byproducts of other pathological processes (e.g., multiple myeloma). Amyloidosis.
  • Chronic graft-versus-host disease: a disorder that typically follows allogeneic hematopoietic transplantation. Can be associated with scleroderma-like skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin changes. Diagnosis is established by skin Skin The skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue. Structure and Function of the Skin biopsy. Treatment involves steroids and immunosuppressive medications.

References

  1. Denton C.P. (2020). Overview of the treatment and prognosis of systemic sclerosis (scleroderma) in adults. Retrieved January 28, 2021, from https://www.uptodate.com/contents/overview-of-the-treatment-and-prognosis-of-systemic-sclerosis-scleroderma-in-adults
  2. Denton C.P. (2019). Pathogenesis of systemic sclerosis (scleroderma). Retrieved January 28, 2021, from https://www.uptodate.com/contents/pathogenesis-of-systemic-sclerosis-scleroderma
  3. Ingegnoli F, Ughi N, Mihai C. Update on the epidemiology, risk factors, and disease outcomes of systemic sclerosis. Best Pract Res Clin Rheumatol. 2018;32(2):223. Epub 2018 Sep 14. 
  4. Jimenez S.A. (2020). Scleroderma. Retrieved January 28, 2021, from https://emedicine.medscape.com/article/331864-overview
  5. Varga J. (2020). Clinical manifestations and diagnosis of systemic sclerosis (scleroderma) in adults. Retrieved January 28, 2021, from https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-systemic-sclerosis-scleroderma-in-adults

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